Lymphatic system dysfunction in pediatric populations
Identifieur interne : 005446 ( Main/Exploration ); précédent : 005445; suivant : 005447Lymphatic system dysfunction in pediatric populations
Auteurs : Christoph M. Papendieck [Argentine]Source :
- Phlebolymphology [ 1286-0107 ] ; 2011.
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English descriptors
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Abstract
Lymphatic system dysfunction in children has many presentations and syndromes and may affect all parts of the body. It can be classified according to the type of abnormality present as dysplasia of the lymphatics (LAD I), dysplasia of the lymph nodes (LAD II), or dysplasia of the lymphatics and lymph nodes (LAAD). Lymphatic dysplasias can be clinically represented by peripheral lymphedema alone or be associated with more and complex dysfunction in different locations, eg, intestinal lymph vessels, thoracic duct, and others. Primary lymphedema has for many years been the least understood form of lymphedema, but exploration of the underlying genetic causes of certain types of primary lymphedema is helping researchers identify and understand previously unrecognized syndromes. Carefully searching for lymphatic dysplasia in these patients, and if indicated in their relatives, as well as establishing the exact nature of the lymphatic dysplasia means that most cases of lymphatic dysfunction can now be diagnosed. This paper summarizes current knowledge on the recognition and classification of lymphatic system dysfunction in pediatric populations, with a focus on primary lymphedema.
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